Childhood IgA nephropathy combined with Alport syndrome: a report of 2 cases and literature review

VernacularTitle:儿童IgA肾病合并Alport综合征2例报告并文献复习
Author: Jieyuan CUI ; Hongwen ZHANG
Keywords: Alport syndrome; IgA nephropathy; child
From: Journal of Clinical Pediatrics 2017;35(1):9-12
CountryChina
Language:Chinese
Abstract: Objective To explore the diagnosis and differential diagnosis of IgA nephropathy.Methods The clinical data of 2 children with IgA nephropathy were retrospectively analyzed.The pertinent literatures were reviewed.Results In 2 males aged 6 and 7 years,the clinical features were a large amount of proteinuria (mainly albumin),low serum albumin,high cholesterol,and persistent microscopic hematuria,which were in line with the diagnosis of nephrotic syndrome.The effects of hormone and immunosuppressive therapy were poor.Renal pathology immunofluorescence and light microscopy findings were in accord with mild to moderate mesangial proliferative IgA nephropathy (M1E0S0T0).Electron microscope showed glomerular basement membrane lesions (layering,breakage,and uneven thickness),which could not exclude Alport syndrome.Further gene detection confirmed a pathogenic mutation of COL4A5.Conclusions It is rare that IgA nephropathy is combined IgA nephropathy at the same time.Attention should by paid to those who had a poor effect of treatment or had a related family history in IgA patients because it is possible that IgA nephropathy and IgA nephropathy may occurred at the same time.